Definition of Paget disease of the breast

Paget disease of the breast: The combination of scaly skin changes of the nipple resembling eczema and an underlying cancer of the breast. The nipple is inflamed because of the presence of Paget's cells. These large irregular cells are themselves not cancerous, but they are almost always associated with a cancer in the breast. The reason for the Paget's cells is still a mystery. In Paget's disease, the nipple and areola (the area surrounding the nipple) are typically red, inflamed and itchy. There may be crusting, bleeding, or ulceration. The nipple may be inverted (turned inwards) and there may be a discharge from the nipple. There is a lump that can be palpated (felt) in the breast in almost half of cases.

Paget's disease of the breast accounts for a small but significant minority (1 to 4%) of all breast tumors. It usually occurs in women in their fifties, but it can occur at a later age. Its occurrence in men is a great rarity. It is sometimes called Paget's disease of the nipple.

The diagnosis of Paget's disease calls for a careful medical history and physical examination and usually a mammogram. The skin changes in Paget's disease can be confused with other skin conditions such as eczema, dermatitis and psoriasis. A biopsy may be done to look for evidence of cancer below the skin surface. The biopsy is usually done using a local anesthetic, which numbs the area. A small piece of skin and underlying breast tissue is taken and sent to the laboratory to be examined under a microscope.

Treatment depends on whether or not the cancer has spread to surrounding breast tissue and also on how much of the breast is affected. Surgery is the main treatment for Paget's disease of the breast. Most people with Paget's disease may require mastectomy. For some people, no further treatment is needed after surgery. Others may receive radiotherapy, the drug tamoxifen or chemotherapy. These may be used separately or together.

The disease takes its name from the great English surgeon and pathologist Sir James Paget (1814-99). At St. Bartholomew's Hospital, London, Paget first reported the association between skin changes of the nipple and the development of breast cancer in 1874. Paget also described a number of other disorders including Paget's disease of bone. He opposed authority and advocated the scientific approach in medicine. Together with his lifelong friend Rudolph Virkow in Germany, Paget is considered one of the founders of modern pathology.